We report a very rare early-stage case of mantle cell lymphoma, which arose from the rectum. A 60-year-old man presented with a small elastically hard polypoid lesion in the rectum. The lesion was 1.2 x 1.2 cm in size. As a preoperatively barium enema and endoscopy suggested a benign tumor of the rectum, he underwent local excision of a rectal polypoid mass transanally under spinal anesthesia. However, histological examination revealed a malignant lymphoma, because the lesion was histologically characterized by solid growths of small to medium-sized round cells. Furthermore, immunohistochemical tests revealed B-cell marker positivity and CD5 positivity, but cyclin D1 negativity. Since it was reported that lymphomas with a mantle cell lymphoma morphology and CD5 expression, but without cyclin D1 overexpression, exist in about 10% of mantle cell lymphoma cases, we diagnosed his disease as mantle cell lymphoma. To our knowledge, this is the first reported case of an early-stage mantle cell lymphoma, originating from the rectum.